Penetrance of the R337H Mutation and Pediatric Adrenocortical Carcinoma Incidence Associated with Environmental Influences in a 12-Year Observational Cohort in Southern Brazil.

The R337H mutation is associated with increased incidence of pediatric adrenocortical tumor (ACT). The different environmental conditions where R337H carriers live have not been systematically analyzed. Here, the R337H frequencies, ACT incidences, and R337H penetrance for ACT were calculated using the 2006 cohort with 4165 R337H carriers living in Paraná state (PR) subregions.

Simulation in Pediatric Video Surgery: Training with Simple and Reproducible Exercises Elaborated by Residents.

Most residents do not have a defined program for simulation training in video surgery in Brazil. The training takes place for the most part or in short courses. The goal of this article is to describe and evaluate a set of exercises using low-cost materials, created by the residents themselves, to enable basic skills training in video surgery.

Impact of neonatal screening and surveillance for the TP53 R337H mutation on early detection of childhood adrenocortical tumors.

Purpose: The incidence of pediatric adrenocortical tumors (ACTs) is remarkably high in southern Brazil, where more than 90% of patients carry the germline TP53 mutation R337H. We assessed the impact of early detection of this mutation and of surveillance of carriers.

Patients And Methods: Free newborn screening was offered at all hospitals in the state of Paraná.

Adrenocortical carcinoma in children: clinical aspects and prognosis.

Although adrenocortical carcinoma is seen infrequently in children in most countries, it is occurs with significant frequency in Southern Brazil. This has enabled a study of the pattern of this disease in the pediatric age group. Of 78 patients with adrenocortical carcinoma seen at two hospitals in Curitiba, Brazil, 55 were adequately documented, with minimum of 2 years' follow-up, and formed the basis of this paper.